Angioimmunoblastic T-Cell Lymphoma and Chronic Lymphocytic Leukemia/Small Lymphocytic Lymphoma
| Autor: | Frédéric Lambert, Manuela Vivario, Philippe Gaulard, Bettina Bisig, Audrey Letourneau, Edoardo Missiaglia, Mounir Trimech, Joan Somja, Laurence de Leval, Monika Nagy-Hulliger, Bernard De Prijck |
|---|---|
| Rok vydání: | 2021 |
| Předmět: |
Angioimmunoblastic T-cell lymphoma
business.industry Chronic lymphocytic leukemia Myeloid leukemia Gene rearrangement medicine.disease Pathology and Forensic Medicine Lymphoma 03 medical and health sciences Leukemia 0302 clinical medicine Immunophenotyping immune system diseases hemic and lymphatic diseases 030220 oncology & carcinogenesis Cancer research medicine Neoplasm Surgery Anatomy business 030215 immunology |
| Zdroj: | American Journal of Surgical Pathology. 45:773-786 |
| ISSN: | 0147-5185 |
| Popis: | Chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) is an indolent small B-cell neoplasm that may transform into a clinically aggressive disease, namely Richter syndrome, usually as diffuse large B-cell lymphoma. Besides, CLL/SLL encompasses an increased risk of developing other secondary cancers, including a variety of T-cell lymphomas, often of the anaplastic large-cell type or with a cytotoxic phenotype. Here, we report a small series of patients with composite lymphomas consisting of CLL/SLL and angioimmunoblastic T-cell lymphoma (AITL), a hitherto unrecognized association. The 3 patients (1 male/2 females, 68 to 83 y) presented with high-grade-type symptoms. One patient was clinically suspicious for Richter syndrome, in the others CLL/SLL and AITL were concomitant de novo diagnoses. CLL/SLL and AITL were admixed in the same lymph nodes (3/3 cases) and in the bone marrow (1/2 cases). In all cases, the AITL comprised prominent clear cells with a strong T follicular helper immunophenotype and similar mutations consisting of TET2 or DNMT3A alterations, IDH2 R172K/M, and RHOA G17V. The 3 patients received chemotherapy. One died of early AITL relapse. The other 2 remained in complete remission of AITL, 1 died with recurrent CLL, and 1 of acute myeloid leukemia. These observations expand the spectrum of T-cell lymphoma entities that occur in association with CLL/SLL, adding AITL to the rare variants of aggressive neoplasms manifesting as Richter syndrome. Given that disturbances of T-cell homeostasis in CLL/SLL affect not only cytotoxic but also helper T-cell subsets, these may contribute to the emergence of neoplasms of T follicular helper derivation. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|